atlas of the newborn volume 1.pdf

8/16/2019 Atlas of the Newborn Volume 1.pdf

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Rudolph

Newborn Atlas of the

V O L U M E 1

Neonataland

Perinatal

Medicine


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V O L U M E 1

Neonataland

Perinatal

Medicine



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Arnold J. Rudolph, M.D.(Deceased)

Professor of Pediatrics

Baylor Medical College

Houston, Texas


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V O L U M E 1

Neonataland

Perinatal

Medicine

Arnold J. Rudolph, M.D.(Deceased)

Professor of PediatricsBaylor Medical College

Houston, Texas

1997

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ForewordSir William Osler stated, “There is no more

difficult task in medicine than the art of

observation.” The late Arnold Jack Rudolphwas an internationally renowned neonatolo-gist, a teacher’s teacher, and, above all, onewho constantly reminded us about how muchcould be learned by simply observing, in hiscase, the newborn infant.

This color atlas of neonatology represents adistillation of more than 50 years of observingnormal and abnormal newborn infants. The Atlas begins with a section on the placenta,its membranes, and the umbilical cord. JackRudolph delighted in giving a lecture entitled

“Don’t Make Mirth of the Afterbirth,” inwhich he captivated audiences by showingthem how much you could learn about thenewborn infant from simply observing theplacenta, its membranes, and the umbilicalcord.

In a few more than 60 photomicrographs,we learn to read the placenta and gain insightinto such disorders as intrauterine growthretardation, omphalitis, cytomegalic inclu-sion disease, congenital syphilis, and congen-ital neuroblastoma. Congenital abnormalities

of every organ system are depicted along withthe appearance of newborn infants who havebeen subjected in utero to a variety of differ-ent drugs, toxins, or chemicals. We also learnto appreciate the manifestations of birth trau-ma and abnormalities caused by abnormalintrauterine positioning.

More than 250 photographs are used toillustrate the field of neonatal dermatology.The collection of photographs used in thissection is superior to that which I have seenin any other textbook or atlas of neonatologyor dermatology; this section alone makes thisreference a required addition to the library of any clinician interested in the care of infantsand children. Photographs of the Kasabach-Merritt syndrome (cavernous hemangiomawith thrombocytopenia), Klippel-Trénaunaysyndrome, Turner’s syndrome, Waardenburg’ssyndrome, neurocutaneous melanosis, mas-tocytosis (urticaria pigmentosa), and incon-

tinentia pigmenti (Bloch-Sulzberger syn-drome) are among the best that I have seen.

Cutaneous manifestations are associatedwith many perinatal infections. The variedmanifestations of staphylococcal infection of the newborn are depicted vividly in photomi-crographs of furunculosis, pyoderma, bullousimpetigo, abscesses, parotitis, dacryocystitis,inastitis, cellulitis, omphalitis, and funisitis.Streptococcal cellulitis, Haemophilus influen-zae cellulitis, and cutaneous manifestations of listeriosis all are depicted. There are numer-ous photomicrographs of congenital syphilis,showing the typical peripheral desquamative

rash on the palms and soles, as well as otherpotential skin manifestations of congenitalsyphilis which may produce either vesicular,bullous, or ulcerative lesions. The variousradiologic manifestations of congenitalsyphilis, including pneumonia alba, ascites,growth arrest lines, Wegner’s sign, periostitis,and syphilitic osteochondritis, are depicted.Periostitis of the clavicle (Higouménaki’ssign) is shown in a photograph that alsodepicts periostitis of the ribs. A beautiful pho-tomicrograph of Wimberger’s sign also has

been included; this sign, which may appear inan infant with congenital syphilis, revealsradiolucency due to erosion of the medialaspect of the proximal tibial metaphysis.

The Atlas also includes a beautiful set of photographs which delineate the ophthalmo-logic examination of the newborn. Lesionswhich may result from trauma, infection, orcongenital abnormalities are included. Thereare numerous photographs of the ocular man-ifestations of a variety of systemic diseases,such as Tay-Sachs disease, tuberous sclerosis,tyrosinase deficiency, and many more.Photographs of disturbances of each of thevarious organ systems, or disorders affectingsuch organ systems, also are included alongwith numerous photographs of different formsof dwarfism, nonchromosomal syndromes andassociations, and chromosomal disorders. Inshort, this Atlas is the complete visualtextbook of neonatology and will provide any


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physician, nurse, or student with a distillationof 50 years of neonatal experience as viewedthrough the eyes of a master clinician.

Arnold Jack Rudolph was born in 1918,grew up in South Africa, and graduated fromthe Witwatersrand Medical School in 1940.Following residency training in pediatrics atthe Transvaal Memorial Hospital forChildren, he entered private pediatric prac-tice in Johannesburg, South Africa. Afteralmost a decade, he left South Africa andmoved to Boston, where he served as a SeniorAssistant Resident in Medicine at theChildren’s Medical Center in Boston,Massachusetts, and subsequently pursued fel-lowship training in neonatology at the sameinstitution and at the Boston Lying-InHospital, Children’s Medical Center andHarvard Medical School under Dr. ClementA. Smith.

In 1961, Dr. Rudolph came to BaylorCollege of Medicine in Houston, Texas, theschool at which he spent the remainder of hiscareer. He was a master teacher, who receivedthe outstanding teacher award from pediatricmedical students on so many occasions that

he was elected to the Outstanding FacultyHall of Fame in 1982. Dr. Rudolph alsoreceived numerous awards over the years fromthe pediatric house staffs for his superb teach-ing skills.

He was the Director of the NewbornSection in the Department of Pediatrics atBaylor College of Medicine for many years,until he voluntarily relinquished that posi-tion in 1986 for reasons related to his health.

Nevertheless, Jack Rudolph continued towork extraordinarily long hours in the care of the newborn infant, and was at the bedside

teaching both students and house staff, aswell as his colleagues, on a daily basis untiljust a few months before his death in July1995.

Although Dr. Rudolph was the author orco-author of more than 100 published papersthat appeared in the peer-reviewed medicalliterature, his most lasting contribution toneonatology and to pediatrics is in the legacyof the numerous medical students, house staff,fellows, and other colleagues whom he taughtincessantly about how much one could learn

from simply observing the newborn infant.This Atlas is a tour de force; it is a spectacularteaching tool that has been developed, collat-ed, and presented by one of the finest clinicalneonatologists in the history of medicine. It isan intensely personal volume that, as Dr.Rudolph himself states, “is not intended torival standard neonatology texts,” but ratherto supplement them. This statement revealsDr. Rudolph’s innate modesty, since with theexception of some discussion on pathogenesis

and treatment, it surpasses most neonatologytexts in the wealth of clinical informationthat one can derive from viewing and imbib-ing its contents. We owe Dr. Rudolph andthose who aided him in this work a debt of gratitude for making available to the medicalcommunity an unparalleled visual referenceon the normal and abnormal newborn infant.

Ralph D. Feigin, M.D. June 13, 1996


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PrefaceI first became attracted to the idea of pro-

ducing a color atlas of neonatology many

years ago. However, the impetus to synthesizemy experience and compile this current col-lection was inspired by the frequent requestsfrom medical students, pediatric house staff,nurses and others to provide them with acolor atlas of the clinical material provided inmy “slide shows.” For the past few decades Ihave used the medium of color slides andradiographs as a teaching tool. In these week-ly “slide shows” the normal and abnormal, aswords never can, are illustrated.

“I cannot define an elephant but I know one

when I see one.”1

The collection of material used has beenadded to constantly with the support of thepediatric house staff who inform me to “bringyour camera” whenever they see an unusualclinical finding or syndrome in the nurseries.

A thorough routine neonatal examinationis the inalienable right of every infant. Mostnewborn babies are healthy and only a rela-tively small number may require special care.It is important to have the ability to distin-guish normal variations and minor findingsfrom the subtle early signs of problems. Thetheme that recurs most often is that carefulclinical assessment, in the traditional sense, isthe prerequisite and the essential foundationfor understanding the disorders of the new-born. It requires familiarity with the widerange of normal, as well as dermatologic, car-diac, pulmonary, gastrointestinal, genitouri-nary, neurologic, and musculoskeletal disor-ders, genetics and syndromes. A backgroundin general pediatrics and a working knowl-

edge of obstetrics are essential. The generallayout of the atlas is based on the above.Diseases are assigned to each section on thebasis of the most frequent and obvious pre-senting sign. It seems probable that the find-ings depicted will change significantly in thedecades to come. In this way duplication has

been kept to a minimum. Additional spacehas been devoted to those areas of neonatal

pathology (e.g., examination of the placenta,multiple births and iatrogenesis) which poseparticular problems or cause clinical concern.

Obviously, because of limitations of space,it is impossible to be comprehensive andinclude every rare disorder or syndrome. Ihave tried to select both typical findings andvariations in normal infants and those foundin uncommon conditions. Some relevantconditions where individual variations needto be demonstrated are shown in more thanone case.

As the present volume is essentially one of my personal experience, it is not intended torival standard neonatology texts, but is pre-sented as a supplement to them. It seemslogical that references should be to standardtexts or reviews where discussion on patho-genesis, treatment, and references to originalworks may be found.

Helen Mintz Hittner, M.D., has been kindenough to contribute the outstanding sectionon neonatal ophthalmology.

I have done my best to make the necessaryacknowledgements to the various sources forthe clinical material. If I have inadvertentlyomitted any of those, I apologize. My mostsincere appreciation and thanks to DonnaHamburg, M.D., Kru Ferry, M.D., MichaelGomez, M.D., Virginia Schneider, PA, and Jeff Murray, M.D., who have spentinnumerable hours in organizing and cullingthe material from my large collection. Wewish to thank Abraham M. Rudolph, M.D.,for his assistance in reviewing the material.

We also wish to thank the following peoplefor their photo contributions to this work:Cirilo Sotelo-Avila, Stan Connor, AvoryFanaroff, Milton Finegold, Brian Kershan,Tom Klima, Susan Landers, Gerardo Cabera-Meza, Ken Moise, Don Singer, EdwardSingleton.


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It is hoped that this atlas will provideneonatologists, pediatricians, family physi-

cians, medical students and nurses with abasis for recognizing a broad spectrum of nor-mal variations and clinical problems as wellas provide them with an overall perspectiveof neonatology, a field in which there contin-ues to be a rapid acceleration of knowledge

and technology. One must bear in mind thecaveat that pictures cannot supplant clinical

experience in mastering the skill of visualrecall.

1. Senile dementia of Alzheimer’s type — normal aging or

disease? (Editorial) Lancet 1989; i:476-477.

Arnold J. Rudolph, M.D.


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CONTENTS

Volume INeonatal and Perinatal Medicine

1. The Placenta, its Membranes, and the Umbilical Cord 1

2. Multiple Births 23

3. Effects of Maternal Medication 47

4. Birth Trauma 57

5. Deformations and Disruptions 81

6. Fetal Growth and Assessment of Gestational Age 117

7. Iatrogenesis 125

Index 154


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Although several texts provide extensive written descriptions of disorders o the newborninfant, the senses of touch, hearing and, especially, sight create the most lasting impressions.

Over a period of almost five decades, my brother Jack Rudolph diligently recorded in pictorialform his vast experiences in physical examination of the newborn. The Atlas of the Newbornreflects a selection from the thousands of color slides in his collection, and truly represents"the art of medicine" as applied to neonatology. A number of unusual or rare conditions areincluded in this atlas. I consider this fully justified because, if one has not seen or heard of acondition, one cannot diagnose it.

In this, the first in a five-volume series, three main topics are covered. Although it is com-mon practice to discard the afterbirth, or placenta and its membranes, careful examination of this fetal organ often provides insight into conditions affecting the newborn. Thus, it mayreveal evidence of intrauterine infection, which may be transmitted to the neonate; of hem-orrhage, which may cause asphyxia; or of vascular or developmental anomalies, which mayresult in intrauterine growth retardation. Many of these placental abnormalities are illustrat-

ed in this volume.Physical forces acting during fetal development, during delivery, or after birth may be

responsible for a variety of anomalies in the newborn. The influences of uterine constraint,of fetal position, and of amniotic bands are demonstrated magnificently, with resulting anom-alies being related to specific fetal postures. Conditions associated with birth trauma, includ-ing fractures, nerve disruptions and other disturbances, are clearly depicted. Many examplesof complications resulting from treatment of the newborn, or iatrogenic problems such as vas-cular complications of umbilical arterial catheterization, are shown graphically.

A major section demonstrates many of the physical anomalies resulting from fetal exposureto various chemicals, such as occurs through maternal drug abuse or administration of phar-macologic agents to the mother during embryonic or fetal development.

This volume will be enormously valuable to obstetricians and neonatologists, as well as tomidwives and nurses involved in the delivery and care of the newborn.

Abraham M. Rudolph, M.D.

Introduction


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Chapter 1

The Placenta, Its Membranes, and theUmbilical Cord

The human placenta is a highly sophisticated organ of interface between mother and fetus, oftenreferred to as the “gate-keeper to the fetus.” Careful examination of the placenta, its membranes,and the umbilical cord can prove to be a valuable aid in the diagnosis and treatment of theneonate. Gross examination of the placenta takes five minutes, and more sophisticated examina-tion should be considered when there is poor pregnancy outcome, recognizable malformations orabnormalities, multiple gestation, extremes of amniotic fluid volume, severe intrauterine growthretardation, short umbilical cord (< 32 cm), and profound acidemia. The maternal surface of theplacenta (decidual plate) is soft, spongy and dark red; and the fetal surface (chorionic plate) isshiny and steel blue to gray. The placenta, membranes, and umbilical cord weigh approximately400 to 600 g at birth. The ratio of fetal to placental size increases with gestation, being less thanor equal to 1:1 at prior to three months, 4:1 at four to six months, and 6:1 at term. Abnormalities

in structure can result in an inefficient transport of oxygen and nutrients to the developing baby.Despite this importance, it is one of the least understood and investigated human organs.

1


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Figure 1.1. A succenturiate (accessory)lobe is common and has no effect on thefetus. This occurs in about 3 to 5% of deliv-eries. Its importance arises from the factthat it may be retained within the uterusand cause postpartum bleeding. (Sotelo-Avila, C.)

Figure 1.2. Another example of a succenturiatelobe. Note that this is very small and the diag-nosis can easily be missed if the placenta is notexamined carefully. (Singer, D.)

Figure 1.3. Fetal surface of a bipartiteor bilobed placenta (placenta du-plex). The two parts of the placentaare of nearly equal size and this occursin about 1% of deliveries. Note thatthe lobes are separated by membranes.The umbilical cord may insert intoone or other lobe, or may insertbetween the two.

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Figure 1.4. A close-up of thesame placenta. The risk to theinfant is that the vessels crossingthe membranes may rupture,resulting in massive blood loss. It issuggested that this condition arisesas a result of superficial implanta-tion of the ovum.

Figure 1.5. Another example of a placenta duplex showing thematernal surface.

Figure 1.6. In a circumvallate (circummarginate)placenta the fetal surface may be reduced if decidualtissue has made its way between the amnion andchorion. This appears as a yellow, peripheral,hyalinized fold circumscribing the edge of the chori-onic plate. This type of placenta has been reportedto be a cause of antepartum bleeding and prematurelabor.

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The Placenta, Its Membranes, and the Umbilical Cord 3


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Figure 1.7. This is an example of placenta mem-branacea (placenta diffusa). These placentas are rare.The ovum implants too deeply, the villae of thechorion fail to regress, and the placental tissuedevelops over the entire surface of the chorion. Theplacenta is very thin and is associated with poor fetalgrowth and antepartum hemorrhage. There may beprevia type bleeding.

Figure 1.8. Transillumination of the same placentashows the thinness of this type of placenta and thatislets of placental tissue are present throughout the

membranes. Pregnancy rarely goes to term and fetaldeath is common. If pregnancy continues to term,placenta accreta may occur. In this condition, there isfailure of separation of the placenta during the thirdstage of labor and there may be severe postpartumhemorrhage.

Figure 1.9. An annular (“girdle” or ring-shaped) is arare form of placenta which resembles a segment of ahollow cylinder. Sometimes a complex ring of placen-tal tissue is seen. More commonly a portion of the ringundergoes atrophy resulting in a placenta which isapproximately horseshoe-shaped. This type of pla-centa is probably a variant of placenta membranacea.Its clinical significance is uncertain but it appears tobe associated with a high incidence of both ante-and postpartum bleeding. The fetus is often small forgestational age. (Connor, S.)

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Figure 1.10. This otherwise normal placenta showsthe presence of an intrauterine contraceptive device,indicating that it did not prevent pregnancy.

Figure 1.11. In premature separation of the placenta(abruptio placentae) there may be massive bleeding of

maternal origin. Note the massive blood loss on theleft of the maternal surface of the placenta. In thesecases, there may be severe fetal asphyxia or death. Theinfant in this case had blood in stool (melena neo-natorum) at birth. This was shown to be ingestedmaternal blood by the Apt test.

Figure 1.12. Another example of abruptio placentae.A large abruptio placentae may result in poor growthof the infant and fetal blood loss.

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Figure 1.13. Fetal surface of a placenta with a largechorangioma (hemangioma of the placenta). Theseinfants may present with severe nonimmune hydropsfetalis. The majority of cases of hydrops fetalis are nowdue to nonimmune causes.

Figure 1.14. Maternal surface of the same placenta. Note the placental enlargement due to the choran-gioma and edema. If the placenta is not examined, thiscause of nonimmune hydrops fetalis may be missed.

Figure 1.15. A calcified, small placenta. This infanthad severe intrauterine growth retardation at term as aresult of poor fetal nutrition.

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Figure 1.16. In velamentous insertion of the cord theumbilical vessels traverse the fetal membranes unsup-ported by either the umbilical cord or by placental tis-sue. If tearing of these unsupported vessels occursbefore or during delivery, it can result in massive fetalblood loss.

Figure 1.17. Another example of velamentous inser-

tion of the cord. Note the vessels traversing themembranes before inserting into the fetal surface of theplacenta. The vessels, lying in loose unsupportedtissue, may easily stretch and tear, especially if theycross the cervical os and result in vasa previa withmassive blood loss. (Sotelo-Avila, C.)

Figure 1.18. An example of velamentous insertion of the cord. Note the large vessels exposed in the mem-branes before they insert into the placental tissue, asection of which is shown at the top. Velamentousinsertion of the cord occurs in 0.5 to 1% of singletonbirths, in 7% of twin births, and in 30 to 40% of tripletbirths. (Sotelo-Avila, C.)

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Figure 1.19. Transillumination of the placenta shownin Figure 1.18. (Sotelo-Avila, C.)

Figure 1.20. Fetus born in a caul. Notethat the membranes completely surroundthe fetus and that the umbilical cord(nuchal cord) encircles the neck twice.A cord around the neck once occurs inabout 20%, and twice in about 2% of pregnancies. Whether the cord causesany problems depends on its tightnessaround the neck. (Klima, T.)

Figure 1.21. Note the petechiae of the face and head and thesubconjunctival hemorrhages in this infant who had a longcord around the neck. The normal umbilical cord is 40 to 60cm long. Long cords (>70 cm) are more apt to be loopedaround the neck or an extremity of the fetus or to have trueknots. Extremely short cords (


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Figure 1.22. This cord was extremely longand wrapped around the left wrist and sev-eral times around the neck resulting inintrauterine death and birth of a stillborn

infant. A long umbilical cord has usuallybeen stretched by the movement of anextremely active fetus.

Figure 1.23. Intrauterine death as a result of a longumbilical cord which wrapped around the neck and thenthe left leg. As the infant moved in utero, he strangledhimself.

Figure 1.24. Along umbilicalcord can encirclean extremity andleave recognizable

grooves (furrows)with or withoutskin ulceration.These are notassociated with apoor outcome. Inthis infant, thecord encircled theright knee verytightly and inter-fered with the cir-culation distally.

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Figure 1.25. The severe umbilical cord constrictionproximally resulted in intrauterine death of this fetus.(Finegold, M.)

Figure 1.26. A hematoma of the umbilical cord(intrafunicular hemorrhage) resulted from a shortumbilical cord. Short umbilical cords are associatedwith extreme intrauterine immobility, such as in thefetal akinesia syndrome.

Figure 1.27. A true knot in the umbilical cord of this

fetus resulted in intrauterine death. The incidence of true knots in the umbilical cord is 0.1 to 1%, and isstrongly associated with long cords and other markersof vigorous fetal activity. It is associated with about10% of stillbirths. The knots must be very tight toobstruct blood flow. At the site of a long standing knot,such as in this fetus, there is a loss of Wharton’s jellyand a constriction of umbilical vessels. Wharton’s jellyprobably prevents umbilical cord blood vessel com-pression by diffusing the pressure exerted by knots. Thejelly is also slippery and this makes it difficult to main-tain a knot.

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Figure 1.28. An example of two true knots in theumbilical cord in an infant who was normal at birth.

Figure 1.29. Close up of the true knot to the right inFigure 1.28. Note that the Wharton’s jelly is normaland that there is no constriction of the cord. This is,therefore, a recent knot. A previously loose knot maybe suddenly tightened as the infant descends duringdelivery.

Figure 1.30. This umbilical cord transection duringamniocentesis occurred several years ago, before ultra-sound was used to determine the position of the amnio-centesis needle. There was considerable blood loss.

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Figure 1.31. Another example of amniocentesisthrough a term placenta. The fetus developed tachy-cardia but was normal at birth. (Moise, K.)

Figure 1.32. Hematoma from transection of theumbilical cord during amniocentesis. There wasmarked blood loss and fetal demise.

Figure 1.33. The diagnosis of single umbilical artery ismade by examining a section through the surface of the umbilical cord. This anomaly is present in 0.7 to1.0% of single placentas and in 3 to 7.0% of multiplebirth placentas. The incidence is low in black infants,but is increased in infants with associated congenitalmalformations. Further investigation is recommendedif a single umbilical artery is associated with one othermajor anomaly.

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Figure 1.34. A histologic section of an umbilical cordwith a single umbilical artery. Note that the thick-walled vessel is the artery and the thin-walled vessel isthe vein. It may be associated with abnormal cordlength, velamentous cord insertion, or circumvallateplacenta. The finding of other congenital malformationsis not specific for any one organ system. Cardiac, renal,gastrointestinal, and skeletal malformities have beendescribed. There is an increased incidence of a singleumbilical artery in trisomy 13 and 18.

Figure 1.35. Note the markedly enlarged,edematous umbilical cord due to excessWharton’s jelly in an infant of a diabeticmother. These cords are very friable and teareasily.

Figure 1.36. A throm-bosed vessel in anumbilical cord with

little Wharton’s jelly.Trauma to the cordis more common whenthere is a lack of Wharton’s jelly. Thelack of Wharton’s jellyis seen more commonlyin postmature infants.A thrombosed vessel inthe umbilical cord maycompromise fetal well-being.

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Figure 1.37. This thin, narrowumbilical cord with total lack of Wharton’s jelly was present atbirth in an infant with postmatu-rity and oligohydramnios. Cordcompression is probably morefrequent with a narrow cord,perhaps because the Wharton’sjelly does not “cushion” the cord.

Figure 1.38. A drying umbili-cal cord 4 days after birth. Notethat as the cord dries, theWharton’s jelly disappearsrapidly.

Figure 1.39. This infant has a large cystin the umbilical cord. The chemicalcomposition of the fluid in this cyst wasthat of serum rather than urine. Thesecysts are thought to arise from the allan-tois.

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Figure 1.40. Another example of an umbilical cord cyst which isthought to arise from the allantois.These cysts are of no clinical signif-icance.

Figure 1.41. Omphalitis andfunisitis in an umbilical cord.Omphalitis is an acute inflamma-tion of the skin surrounding theumbilicus. Funisitis is an acuteinflammation of the umbilicalcord itself. It results from bacteria

or mycoplasma in the amnioticfluid attracting fetal neutrophilsto migrate out of the umbilicalcord vessels. It can be associatedwith necrosis and calciumdeposits within the cord.

Figure 1.42. Histologic sectionof an umbilical cord showingfunisitis. Note the markedinflammatory reaction surround-ing an artery in the cord itself.(Sotelo-Avila, C.)

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Figure 1.43. Histologic section of an umbilical cord showing markedinflammatory reaction and bacterialcolonies (intensely stained blueareas) surrounding a vein in thecord itself.

Figure 1.44. Histologic sec-tion of the umbilical cord in aninfant with congenital listerio-sis. Note the gram positiveorganisms on the surface of theumbilical cord.

Figure 1.45. Histologic sectionof funisitis occurring as a resultof a catheter in situ in an umbil-ical vessel.

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Figure 1.46. The external surfaceof the umbilical cord shows cheesywhite areas similar to thrushwhich suggest the diagnosis of congenital candidiasis. (Sotelo-Avila, C.)

Figure 1.47. A histologic sectionof the umbilical cord of the sameinfant showing funisitis with thehyphae of Candida which arestained pink. (Sotelo-Avila, C.)

Figure 1.48. External surface of anumbilical cord with yellowish brownareas on the external surface whichsuggest the diagnosis of congenitalcandidiasis. (Fanaroff, A.)

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Figure 1.49. Fetal surface of aplacenta showing chorioamnioni-tis. Normally, the fetal surface of aplacenta is shiny and clear with aprominent vascular pattern. Inchorioamnionitis the fetal surfaceappears dull and opaque with anobscure vascular pattern. Theamniotic fluid is cloudy, and theplacenta, membranes, and amni-otic fluid may have a foul odor.Aspiration in this infected milieuby the fetus may result in neona-tal pneumonia, sepsis and/orneonatal meningitis.

Figure 1.50. Histologic sec-tion of the placenta showingchorioamnionitis. Factorsassociated with increased riskof placental infection includepremature and prolonged rup-

ture of the membranes,prolonged labor, placentaprevia, and multiple births.Bacterial infections are morecommon than viral and fungalinfections.

Figure 1.51. Histologic sectionof a placenta with cyto-megalovirus infection. Note thetypical “owl’s eye” inclusions inthe villae. Viral agents reach thefetus by hematogenous dissemi-nation and traverse the placentalvillae. Since viral lesions tend tobe microscopic, gross examina-tion of the placenta is not help-ful. (Finegold, M.)

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Figure 1.52. Histologic section of a placenta with congenital syphilis.

Note the numerous spirochetes.(Finegold, M.)

Figure 1.53. The presence of meconium staining of the

amniotic fluid occurs with fetal distress and postmaturi-ty. Meconium staining of the placenta may occur with-in 1 hour. In cases of chorioamnionitis, the fetal surfacemay be green; in listeriosis, there may be brown orchocolate staining of the amniotic fluid with similarstaining of the placenta. (Finegold, M.)

Figure 1.54. Infant with alarge “garment” nevus (bathingtrunk nevus). Note the markedbreakdown and ulceration of the skin.

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Figure 1.55. Gross section of placenta show-ing multiple nevi in the same infant shownin Figure 1.54. This emphasizes the impor-tance of examining the placenta in infantsborn with such abnormalities.(Sotelo-Avila, C.)

Figure 1.56. Histologic section of

melanoma cells from the placentaof the same infant shown in Figure1.54 and 1.55. Similarly lesionscan be seen in the placenta of infants with congenital neuroblas-toma. (Sotelo-Avila, C.)

Figure 1.57. Squamous metaplasiaof the amnion. In this placenta withsquamous metaplasia, the amnion isstripped from the membranes. Notethe concentric appearance of thesenodules and their frequent umbilica-tion. The amnion is squamous andthese areas of metaplasia form withmaturity. Note the tiny nodules of keratin irregularly present on thefetal surface.

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Figure 1.58. Histologic section of the amnion showing the squamousmetaplasia. Squamous metaplasiadiffers from amnion nodosum inthat the lesions cannot be separatedreadily, whereas the nodules inamnion nodosum can be picked off of the underlying amnion leaving asemi-transparent, saucer-shaped de-pression with somewhat raggededges.

Figure 1.59. Typical amnionnodosum in one twin. This twin wasan acardiac monster having no uri-nary tract. The amnion is rough andshows numerous fine granules which

are whitish, opaque, and uniform insize. Frequently the nodules are moreirregular in size and slightly yellowishbrown. It is unusual to see this degreeof amnion nodosum. The other twinwas normal.

Figure 1.60. Another example of amnion nodosum in a twin placenta.

Note the normal umbilical cord andplacenta on the right and the smallumbilical cord with a thrombosed ves-sel on the left. This was a twin-twintransfusion syndrome and the twin onthe left became a fetus papyraceus.The amnion nodosum lesions arewhitish, opaque, and uniform in size.

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Figure 1.61. Histologic section of a pla-centa and membranes showing amnionnodosum on the left and chorioamnioni-tis on the right in an infant with renaldysgenesis and hypoplastic lungs.

Figure 1.62. High-power histo-logic section of amnion nodosum

which consists of lanugo, vernixcaseosa, and squamous epithelialcells. With marked oligohydram-nios, the fetal skin rubs against thefetal surface of the placenta andproduces these lesions.

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Chapter 2

Multiple BirthsMultiple gestation occurs frequently in pregnancy. The most common is twinning, whichoccurs in about one in every 80 pregnancies. There are two types of twins: monozygous or

“identical” and dizygous or “fraternal.” The monozygous twin rate is 1 in every 200 pregnan-cies. It results from a single ovulation with subsequent splitting of the developing egg withinthe first 14 days. There is no familial tendency. Dizygous twinning results from doubleovulation and fertilization and is probably determined by higher gonadotropin secretion rates.The rate of dizygous twinning is variable and is influenced by heredity (transmitted autoso-mally but expressed only in the mother), race (as high as 1 in 23 births in some West Africanraces; as low as 1 in 300 births in Mongolian races), maternal age (increased frequency withincreasing age), and drugs (incidence of twinning is 6.6% with the use of Clomid®).Examination of the placenta of multiple births is important because of the two- to three-foldhigher incidence of structural defects in monozygous twins and increased potential for suc-cessful future transplant between monozygous twins. Monochorionic placentas are invariably

fused and about 75% of the infants are identical twins. Dichorionic placentas may be fused orseparate. Infants with a dichorionic fused placenta may be identical (monozygous) or fraternal(dizygous). The membrane which separates the two fetal cavities is the key to the evaluationof twin placenta. Typically, this runs across the middle of the fused placenta. Inmonochorionic twins, with one chorion covering two amnia, the dividing membrane consistsof two translucent amnionic layers which can be pulled with ease from the placental surface.In 1% of monozygotic twinning, the placenta will be monochorionic-monoamnionic. Inconjoined twinning, the placenta is also monochorionic-monoamnionic. In dichorionictwins, with two amnia, the dividing membrane consists of four layers, two chorionic and twoamnionic; it is opaque, thicker, and does not separate from the placental surface without tearing.

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Figure 2.1. Theseinfants are normal,identical triplets bornat 32 weeks gestation.They initially hadmild respiratory dis-tress, but improvedrapidly. (Cabera-Meza, G.)

Figure 2.2. The fetal surfaces of the placentas of fraternal triplets.

Figure 2.3. A triplet placenta showing a vela-mentous insertion of the cord in one of thetriplets. Note that the placentas are fused andthus the pregnancy could be monozygotic.

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Figure 2.4. The fetal sur-faces of two completelyseparated twin placentas.These are always dizygotic.

Figure 2.5. This low-powerhistologic section demonstratesthat an amnion can be identi-fied on each side of two choria.Thus, this is an example of a

dichorionic-diamnionic pla-centa. This type of dividingmembranes is typically seen inall dizygotic twinning and inabout 20 to 25% of monozy-gotic twinning.

Figure 2.6. The fetal surface of thisfused, twin placenta shows the divid-ing membranes. In this placenta, his-tologic examination showed thatthere were two amnia and a singlechorion (diamnionic-monochorion-ic). These twins would thus bemonozygotic.

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Figure 2.7. Histology of the mem-branes of the same placenta shows thedouble amnion on the outer surfaceswith a single chorion (diamnionic-monochorionic).

Figure 2.8. On the fetal surface of

this fused, twin placenta, the dividingmembrane is very thin and clear, sug-gesting that this is a monozygoticpregnancy. This was confirmed by his-tologic examination showing a singlechorion. (Finegold, M.)

Figure 2.9. The histologicappearance of the dividing mem-branes in twin placentas are com-pared in this figure. On the left,note the diamnionic-monochori-onic twin placenta, and on theright, note the diamnionic-dichorionic twin placenta.

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Figure 2.10. Note the two umbilicalcords inserting into the fetal surfaceof the placenta without the presenceof dividing membranes. This is typicalof a monoamnionic-monochorionictwin placenta, which occurs in 1% of twin pregnancies.

Figure 2.11. The fetal surface of amonoamnionic-monochorionic twinplacenta, showing the insertion of two separate umbilical cords. (Sotelo-Avila, C.)

Figure 2.12. Close-up of theabove placenta shows anastomosisof the vessels between the twoumbilical cords. This results in atwin (feto-fetal) transfusion syn-drome. (Sotelo-Avila, C.)

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Figure 2.13. The fetal surface of a monoamnionic-monochorionic twin placenta showing the entan-glement of the two umbilical cords resulting in fetalanoxia and fetal distress with meconium passage.

Note the meconium-stained appearance of the fetalsurface of the placenta. Entanglement of the cordsis a complication which may occur because of a lackof a dividing membrane in monoamnionic-mono-chorionic twins. (Karishan, B.)

Figure 2.14. Another exam-ple of entangled cords in amonoamnionic-monochorion-ic twin placenta.

Figure 2.15. The fetal surface of this monoamnionic-monochorion-ic placenta in conjoined twinsshows the insertion of the twoumbilical cords, but note thatthese fuse and present as a singlefused cord in thoracopagus twins.(Sotelo-Avila, C.)

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Figure 2.16. The appearance of the umbilicalcords in a twin pregnancy. Note the small sizeof the umbilical cord in the infant who wasgrowth-retarded as compared to the normalsize of the umbilical cord in the normal twin.

Figure 2.17. These infants born as stillbirthsat 30 weeks gestation are an example of twin(feto-fetal) transfusion syndrome. In this syn-drome, vascular anastomoses permit the trans-fer of arterial blood under high pressure fromthe twin on the right (donor) to the lowpressure venous system of the other twin(recipient). The donor twin is thus kept hypo-volemic, dehydrated, malnourished, or even in

shock. His organs are small and his amnioticfluid is decreased. The recipient twin becomeshypervolemic, edematous, and plethoric (poly-cythemic). His organs are large, he may havecongestive failure and his amniotic fluid isincreased.

Figure 2.18. These monozygotic twins at birth repre-sent another example of twin transfusion syndrome.The birth weight of the twin on the left was 3000 gwith a central hematocrit of 86%. The birth weight of the twin on the right was 2230 g with a central hema-tocrit of 27%. Twin transfusion syndrome occurs onlyin monochorionic twins. It occurs in 15 to 30% of monochorionic pregnancies and is defined in terms of a difference of greater than 250 g birth weight and/or20% difference in the central hematocrit between thetwins. Twins in this syndrome usually do not look iden-tical at birth although in fact they are monozygotic.

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Figure 2.19. In these infants with twintransfusion syndrome, the difference inbirth weight was only 180 g and the dif-ference in central hematocrit was 32%.Thus, these infants represent a mildexample of twin transfusion syndrome.

Figure 2.20. The maternal surfaceof a monozygotic twin placenta ininfants who had the twin transfusionsyndrome. Note on the left the con-gested appearance of the portion of the placenta supplying the recipient

twin who had a central hematocritof 69%, and note on the right themuch paler appearance of theplacenta supplying the donor twinwho had a central hematocrit of 45%. (Singer, D.)

Figure 2.21. The fetal surface of

the same placenta as in Figure.2.20 showing the congestedappearance on the left of therecipient portion of the twin pla-centa and the pallor on the rightof the donor portion of the twinplacenta. These vascular connec-tions may be artery-to-artery,vein-to-vein, or artery-to-vein.Any form may significantly affectthe fetuses physiologically andclinically.

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Figure 2.22. The fetal surface of placentas in twin transfusion syn-drome showing the anastomosesbetween the two fetal circula-tions following the injection of infant formula, which is used as acontrast medium. Note that onthe fetal surface of the placentaarteries always cross over veins.The anastomosis between anartery on the left and a vein onthe right is shown at the junctionof the left third and the righttwo-thirds of the figure.

Figure 2.23. This close-up viewclearly shows the artery-to-veinanastomosis.

Figure 2.24. Another exampleof an injection of infant formulafilling the arteries which crossover the veins in this vessel anas-tomosis.

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Figure 2.25. In extreme cases the transfusion syn-drome may cause the death of one twin resulting in afetus papyraceus. In this figure, there is an extrachorialplacenta with a fetus papyraceus attached.

Figure 2.26. Close-up of the extrachorial placentawith the fetus papyraceus attached.

Figure 2.27. Another exampleof the twin transfusion syndrome.

Note the maternal surface of theplacenta showing the congestedrecipient placenta on the left andthe pale donor placenta on theright with an attached fetuspapyraceus.

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Figure 2.28. The fetus papyraceus may calcify and result in alithopedion (“stone child”) as shown in this figure.

Figure 2.29. A stillborn maleinfant with a birthweight of 1170 gand length of 34 cm is an exampleof acardius acephalus. This is one of the acardiac anomalies whichoccurs as a consequence of abnor-mal umbilical artery-to-artery anas-tomoses between two fetuses in thepresence of a fused placenta. It issometimes referred to as the TRAP(twin-reversed-arterial- perfusion)sequence. (Klima, T.)

Figure 2.30. A close-up of the abdomen of the sameinfant shows an omphalocele; there was also hydrops,pulmonary agenesis, and polyhydramnios. Acardiacanomalies include three groups: acephalus in 60 to 75%of cases, amorphus in 20% of cases, and awell-formed head and body in 10% of cases. (Klima, T.)

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Figure 2.31. The fused placenta of the same infant with acardiusacephalus shown in Figure 2.29 and2.30. Note the large cord of the“normal” infant which had threevessels and the small cord of theinfant with acardius acephaluswhich had two vessels. There wereno separating membranes betweenthe two cords. Note the large anas-tomoses between the placentas.(Klima, T.)

Figure 2.32. An acardiusacephalus infant deliveredat 30 weeks gestation. Theupper body and shoulders

form a fleshy mass cappedby a tuft of short hairs.The lower body has twowell-formed legs but hasclubbed, bifid feet with twotoes. (Klima, T.)

Figure 2.33. Radiographof the infant with acar-dius acephalus. Note the

lack of cranial develop-ment. Two clavicles andscapulae are present. Thevertebral column is nor-mal. The chest is narrowdue to lack of the heartand hypoplastic lungs.The pelvis and lowerextremities are well min-eralized and relativelynormal except for lack of some metatarsal bones.

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Figure 2.34. The twin withthe dominant heart of thesame pregnancy often showsadditional anomalies such aslimb reduction defects. Thesehave been attributed to em-bolic disease consequent uponstasis in the acardiac fetus.This twin had some limbanomalies which includedfusion of digits of the left handas seen in this figure.

Figure 2.35. This isthe same infant show-ing anomalies of bothfeet.

Figure 2.36. The placenta of the infants described aboveshowing two umbilical cordswith a common insertionabout 5 cm from the margin

of the placenta. The largercord was approximately 10mm in diameter and hadthree vessels. The smallercord was approximately 6 mmin diameter and had two ves-sels. Note the congestedappearance of the placentaltissue and the vessels on theleft and the pallor of the pla-cental tissue and the vesselson the right. (Klima, T.)

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Figure 2.37. This is an example of acardius anceps (hemiacardius). Thecranial portion is partially covered byhair, but no head is formed. There is aglobular structure of the upper partwhich shows a rudimentary face withan oral opening through which a cleftpalate is seen. Four extremities are pre-sent. There is a defect in the anteriorabdominal wall with intestinal loops

present. (Klima, T.)

Figure 2.38. Close-up of the same infantshown in Figure 2.37. The anterior wallof the abdomen is missing and theintestinal loops are exposed. There is noumbilical cord identified. (Klima, T.)

Figure 2.39. In thoracopagus conjoined twins, note the fusion at the thorax andupper part of the abdomen with a single site of umbilical cord insertion. Theposture is typical for thoracopagus conjoined twins in that the heads are hyper-extended and the backs are relatively straight.

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CONJOINED TWINSConjoined twins are rare, one per 50,000 to 100,000 live births. They result from the failure of the zygote to com-pletely divide. This occurs in approximately 1% of monozygotic twins. The incidence of the types of fusion is asfollows: The most common types are thoracopagus twins with an incidence of one per 70,000 live births (73.4%),pygopagus twins (18.8%), ischiopagus twins (5.9%), and the most rare types are the craniopagus twins withan incidence of one per 2,000,000 to 4,000,000 live births (1.7%). Conjoined twinning occurs only in mono-amnionic-monochorionic pregnancies.


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Figure 2.40. Radiograph of the conjoined twins shown inFigure 2.39 illustrates the hyperextended heads and thefusion of the thoraces and upper abdomen. On fetal radi-ograph, the hyperextended fetal heads at the same level

were considered almost diagnostic of thoracopagus con-joined twins. With the advent of ultrasonography, the diag-nosis should be made more readily.

Figure 2.41. Another set of thoracopagusconjoined twins showing the typical pos-ture of the hyperextended heads.

Figure 2.42. Thoracopagus conjoined twins showing the fusion from theupper thorax to the midabdomen.

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Figure 2.43. The same twins as shown in Figure 2.41showing the fused upper abdomen and the fusedumbilical cord.

Figure 2.44. This fused umbilical cord section fromthe same set of thoracopagus conjoined twins showsthe presence of four vessels, two arteries and two veins.In the fused umbilical cords seen in conjoined twin-ning, there may be from two to seven vessels. (Singer, D.)

Figure 2.45. This fused umbilical cord section show-ing three arteries and three veins is another examplefrom conjoined twins. (Singer, D.)

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Figure 2.47. The same set of pygopagus conjoined twinsshowing the fused genitalia.

Figure 2.48. Close-up of the fused genitalia in thesame set of pygopagus conjoined twins.

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Figure 2.46. Pygopagus conjoined twins are fused at thebuttocks.


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Figure 2.49. The twins shownin Figure 2.46 were success-fully separated. This figureshows them prior to dischargefrom the hospital.

Figure 2.50. Radiograph of a set of omphalopagus twins shows the fusion at theabdominal walls and hence they could beseparated.

Figure 2.51. An exam-ple of craniopagus con-joined twins.

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Figure 2.52. Note on the left, the vaginal delivery of prosopothoracopagus conjoined twins. On the right, notethe twins following delivery. Prosopothoracopagusconjoined twins are twins united in the frontal plane withthe fusion extending from the oral region through thethorax and upper abdomen. Diagnosis was not made pre-natally and the twins died at delivery. Note that there areonly two upper extremities (dibrachus) and four lowerextremities (tetrapus). (Caberra-Meza, G.)

Figure 2.53. Cephalothoracopagusconjoined twins. Note the syncephalus.

Figure 2.54. Dicephalus con-joined twins with dicephalus,three upper extremities (tribra-chus), and two lower extremi-ties (dipus). Note the two sepa-rate heads and the fused chest.

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Figure 2.55. A close-up of the same twins shown inFigure 2.54 demonstrates the third upper extremityextending from the fused shoulders. There were twoseparate neurologic systems, two separate pulmonarysystems, two gastrointestinal systems joining at the

jejunum, a single genitourinary system, and conjoinedhearts with complex anomalies.

Figure 2.56. Another example of di-cephalus conjoined twins. Note that thehead on the left of the figure is normal butthat there is anencephaly of the head onthe right of the figure.

Figure 2.57. Radiograph of the same conjoined twinsshowing the normal head onthe left of the figure and theanencephalic head on theright of the figure. Note thatthere are fused vertebralcolumns with some separa-tion at the lower thorax andupper abdomen and thatthere is dibrachus.

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Figure 2.58. Asymmetric ischiopagus conjoinedtwins. Note that the twin on the right of the figure,which is attached at the ischia, is an anencephalicparasite with a partial thorax and abdomen and twoupper and two lower extremities. The “normal” twinon the left of the figure had gastroschisis, imperforateanus, and rectovaginal fistula. There were twobladders, two kidneys, and two uteri present.

Figure 2.59. Close-up of the anencephalic parasitein the above asymmetric ischiopagus conjoined

twins.

Figure 2.60. Radiograph of the asymmetric ischiopagusconjoined twins. Note at the bottom the severelyanecephalic twin attached to the “normal” twin who hasa large gastroschisis. Each twin has four extremities.

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Figure 2.61. Preoperative (above) and postoperative(below) appearance of the asymmetric ischiopagusconjoined twins shown in Figures 2.58 to 2.60.

Figure 2.62. Dipygus twins. Recent work hassuggested that genes are involved in establish-ing the body axes, metameric pattern (seg-mentation genes), and regional specialization(homeotic genes). This raises the possibilitythat many of the abnormalities of facial dupli-cation in man are not a manifestation of incomplete twinning but may be homeoticmalformations. Limb duplication may also bea result of stimulation of homeobox geneexpression and thus a well-formed pair of armsand/or legs may be seen emerging.

Figure 2.63. Thisinfant has a well-developed lower ex-tremity emerging fromthe chest and hasbeen considered to be

an asymmetrical dou-ble malformation –heteroadelphia (anunderdeveloped para-site attached to a well-developed autosite).With the new con-cept, this could bean example of a ho-meotic malformation.Also note the largeomphalocele.

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Figure 2.64. Close-up view of thesame accessory lower extremity asshown in Figure 2.63.

Figure 2.65. Duplication of the right legand foot. The question again arises as towhether this is an asymmetric double mon-ster or a homeotic malformation. (Cabera-Meza, G.)

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Figure 2.67. Another exam-

ple of discordant twins. Thetwin on the left is an albinoand the twin on the right isnormal. This is the second setof discordant twins (one albi-no and one normal) born tothis mother.

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Figure 2.66. Discordanttwins. The twin on the left isan example of a severe caudalregression syndrome. Therewas oligohydramnios, renalagenesis, imperforate anus,and lack of external genitalia.The twin on the right is nor-mal. (Cabera-Meza, G.)

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Chapter 3

Effects of Maternal MedicationDuring pregnancy, the average fetus is exposed to four physician-prescribed and fiveself-prescribed drugs. Every drug administered or taken by a pregnant woman presents the

mother with both risks and benefits. The risks include the drug’s potential as a teratogen oras a cause of toxicity in the fetus. Most human teratogens affect the embryo during a very nar-row period of early development as illustrated by the time (24 to 33 days gestation)during which the fetus is susceptible to limb reduction defects caused by thalidomide. Severalhuman teratogens, such as alcohol, androgens, cocaine, diphenylhydantoin, radiation,tetracycline, valproic acid, and warfarin have serious side effects beyond the period of organogenesis. These effects may include cell deletion, vascular disruption, necrosis,physiologic decompensation, organ pathology, and intrauterine growth retardation. Drugstaken in the third trimester may not have teratogenic effects, but may be toxic to the fetus.Some examples include indomethacin (causing oligohydramnios), propylthiouracil (causingfetal goiter), and erythromycin (causing cholestatic hepatitis). A detailed history of mater-

nal drug use and abuse is essential in evaluating most malformations and diseases in theneonatal period.

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Figure 3.1. This illustration contrasts thecraniofacial features of a healthy child on theright to those of a child with fetal alcoholsyndrome on the left. Note the microcephaly,short palpebral fissure, flat maxillary area,poorly developed philtrum and thin upper lip(Peter Shvartsman, Canadian MedicalAssociation Journal, July 15, 1981 cover).

Figure 3.2. This infant, age 6 weeks, was bornto a mother with severe, chronic alcoholism.There was failure to thrive and hypotonia. Notethe microcephaly (head circumference less thanthe third percentile), short nose, absence of

philtrum and thin vermilion border of theupper lip.

Findings in fetal alcohol syndrome includeintrauterine growth retardation, microcephaly,dysplastic facial features, hypoplasia of themidface, and a hypoplastic philtrum with a thinvermilion border of the upper lip. Later theremay be continued failure to thrive and develop-mental and behavioral disorders.

Figure 3.3. Close-up of the face of the sameinfant shows the short nose, absence of thephiltrum, and thin vermilion border of theupper lip. Many other findings in fetal alcoholsyndrome have been reported, including epican-thic folds, ptosis, hypoplastic maxilla, deep oraccentuated palmar creases, and clinodactyly.

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Figure 3.4. Soon after birth, this infant of anarcotic addict shows hypotonia. Note theconcavity of the inner aspect of the thighs andthe position of the lower extremities. This hasresulted from a postural deformation in whichthe fetus has had its thighs flexed over itsabdomen in utero. Because of the mother’snarcotic habit there was minimal fetal move-ment in utero.

Figure 3.5. Drug withdrawal is a major problem inneonates delivered of narcotic addicted mothers. Thisfigure stresses the fact that one should always check for

signs of drug addiction in the mother. This figure showsneedle tracks at both elbows of a mother.

Figure 3.6. Infants withretinoic acid embryopathy(Accutane™ embryopathy)may have craniofacial, cardio-vascular, and central nervoussystem abnormalities.In this infant note the nar-row sloping forehead, flat

depressed nasal bridge, mildmicrognathia, and microtiawith absence of the externalauditory canal. In additionthere was congenital heartdisease. Affected infants mayhave hydrocephalus, micro-cephaly, or thymic abnor-malities. This mother wastreated with retinoic acidduring the first month of pregnancy.

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Figure 3.7. Close-up of the ears of thesame infant as shown in Figure 3.6 showsthe bilateral microtia with absence of the external auditory meatus.

Figure 3.8. In infants with thefetal hydantoin (Dilantin™) syn-drome there is moderate growthretardation, usually prenatal, awide anterior fontanelle andmetopic ridging. In this infant,

note the growth retardation, pro-fuse scalp hair, and short neck.Other findings included hypopla-sia of the distal phalangeswith small nails and a digitalthumb.

Figure 3.9. Close-up of the face of the same infant. Note the marked hir-sutism, low hairline, low nasal bridge with a short upturned nose (“pug” nose),and long philtrum.

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Figure 3.10. Hypertrichosis inanother infant with the fetalhydantoin syndrome. Motherwas treated throughout pregnan-cy with hydantoin. The risk of fetal hydantoin syndrome ininfants of treated mothers isabout 10%.

Figure 3.11. Gum hypertrophyin an infant with the fetal hydan-toin syndrome. Many other find-

ings have been reported in infantswith fetal hydantoin syndrome,including widely spaced nipples,rib anomalies, abnormal palmarcreases, pilonidal sinus, and con-genital heart disease.

Figure 3.12. This infant of an epileptic mother on hydantoindeveloped seizures at the age of 36 hours. He had hypocalcemia with acalcium level of 6.4 mg/dL and a phosphorus level of 11.2 mg/dL. Infetal hydantoin syndrome the digital hypoplasia may be associated withnarrow distal phalanges and hypoplastic nails.

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Figure 3.13. This infant with thefetal hydantoin syndrome presentedwith many of the findings alreadydescribed. There was growth retarda-tion, hypertelorism, small pug nose,anteverted nostrils, long philtrum,and thin vermilion border of theupper lip, and short neck.

Figure 3.14. The same infant shows the characteris-tic changes in the fingers. Note the hypoplasia of thedistal phalanges with hypoplastic or absent nails andthe digital thumbs. There is mild webbing.

Figure 3.15. The same infantwith fetal hydantoin syndromeshows the marked hypoplasia of the distal phalanges of the toesand absent or hypoplastic nails.

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Figure 3.16. Postnatal growthdeficiency and microcephaly arepresent in two-thirds of childrenexposed to valproic acid incombination with other anticon-vulsants. It does not occur withmonotherapy with valproic acid.This infant with the fetal vaproatesyndrome shows the typicalcraniofacial abnormalities. Notethe trigonocephaly with a promi-nent metopic ridge, bifrontalnarrowing, outer orbital ridgedeficiency, midface hypoplasia,epicanthic folds, small shortupturned nose, and long flatphiltrum.

Figure 3.17. A cranial view of the sameinfant shows the trigonocephaly due topremature closure of the metopic suture,bifrontal narrowing, and outer orbital ridgedeficiency.

Figure 3.18. Lateral view of thehead and face of the same infantshows the marked metopic ridge,small flat short nose, micrognathiaand “square” ears.

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Figure 3.19. The sameinfant with fetal valpro-ate syndrome as shownin Figures 3.16 to 3.18,had distal phalangealhypoplasia and taperingof the fingers. Notethe abnormal creaseson the fingers and palmdue to lack of fetalmovement in utero.Other changes reportedin infants with this syn-drome include tracheo-malacia, congenital heartdefects, and urogenitalanomalies.

Figure 3.20. Yellow staining of the teeth in a child exposed tomaternal tetracycline in utero.

Figure 3.21. A Wood’s filter showsthe fluorescence of the nails in aninfant exposed to maternal tetracy-cline. If young infants are giventetracycline after birth the stainingof the teeth and nails also occurs.

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Figure 3.22. Drug-induced pseudoher-maphroditism in a female infant who wasvirilized by progestational agents during thefirst trimester of pregnancy. The incidenceof this condition has decreased because,with recognition of this iatrogenic cause of virilization of the fetus, there has been adecreased use of incriminating drugs such asprogestational agents or androgens duringthe first trimester. There may be fusion of labioscrotal folds with formation of a uro-genital sinus and clitoromegaly. (SeeVolume V, chapter 5).

Figure 3.23. The thalidomide syn-drome in twin infants born to a motherwho took thalidomide early in gestation.

Maternal ingestion of thalidomidebetween the 25th to 44th day after con-ception may cause malformations. Inthe thalidomide syndrome the limbs areusually asymmetrically involved and themalformations of the extremities are of all grades of severity (digits are usuallypresent). There may be microph-thalmia, ear deformities, and cardiac,renal and intestinal malformations.

Figure 3.24. Phocomelia inanother infant born to anoth-er mother who took thalido-mide in early gestation. Notethe asymmetric phocomelia.

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Figure 3.25. This infant with the fetal warfarin syndrome (Coumadin™embryopathy) was born to a mother who was being treated with warfarin dur-ing the first trimester of pregnancy. These infants typically are low birth-weight and have facial and skeletal abnormalities. Less commonly they mayhave central nervous system and eye abnormalities. In this baby note the typ-ical facial features of a broad flat face and nasal hypoplasia with a low nasalbridge, a prominent philtrum, and micrognathia.

Figure 3.26. The lateral view of theface strikingly demonstrates the

marked nasal hypoplasia resulting ina very flat face. Because of themarked nasal hypoplasia theseinfants often present with upper air-way obstruction.

Figure 3.27. Radiograph of the lower extremities of thesame infant shows the stippling of the epiphyses at the prox-imal femora. Stippling of the epiphyses may occur along thevertebral column and the tarsal bones. The stippling disap-pears in the first few years of life. Coumadin™ embryopathy isphenotypically similar to hereditary chondrodystrophiapunctata and it must thus be distinguished from the differenthereditary forms of Conradi-Hünermann syndrome.

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Chapter 4

Birth TraumaBirth trauma refers to those injuries sustained during labor and delivery. Despite skilled and com-petent obstetric care, some may be unavoidable. Factors predisposing infants to injury include

macrosomia, prematurity, cephalopelvic disproportion, dystocia, prolonged labor, and abnormalpresentation. In 1988, birth injuries ranked eight as major causes of neonatal mortality and caused4.6 deaths per 100,000 live births. The clinician who cares for newborn infants must be familiarwith the conditions caused by birth injury. Injuries are known to occur to the soft tissues, head,eyes, ears, vocal cords, neck and shoulder, spine and spinal cord, intra-abdominal organs, extrem-ities, and genitalia. Although many are mild and self limited, others are serious and potentiallylethal.

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Figure 4.1. Severe molding of the head following anoccipitoposterior presentation. The mobile skull bonesand brain deform to comply with pressure in the birthcanal. Note the flattened forehead and long occiput. Itresolves spontaneously and needs no intervention.

Figure 4.2. Persistent occipitoposterior presentationwith marked molding and a caput succedaneum. A

caput succedaneum occurs as a result of the presentingpart pressing against the partly dilated cervix whoseconstricting rim obstructs the return flow of venousflood and lymph from the scalp leading to edema. Thedistribution crosses suture lines (compare with cephal-hematoma) and is usually present at birth.

Figure 4.3. Caput succedaneum with prolonged labor.In a caput succedaneum the tissues involved are thoseencircled by the “girdle of contact” formed by thematernal passages. The location indicates theintrauterine lie of the fetus. In breech delivery theremay be similar edema and bruising of the perineum,buttocks, or genitalia. These are really an equivalent of caput succedaneum.

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Figure 4.4. “Blisters” of the skin following prolongedlabor over a caput succedaneum in an infant at the ageof 1 day following prolonged labor. Aspiration of thematerial was sterile.

Figure 4.5. A large caput (chignon) following vacuum

extractor delivery. The word “chignon” refers to thelocalized area of scalp edema caused by the suction of the cup of the vacuum extractor. Most cases resolvespontaneously but if associated with perinatal asphyxiathere may be necrosis of the chignon leading to ulcer-ation of the scalp.

Figure 4.6. “Caput ring.” In rare cases with persistentstrong contractions and a slowly dilating cervix, necro-sis of the scalp may occur in the area of a caput due topressure ischemia occurring during a prolonged labor.

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Figure 4.7. Cephalhematoma over the right parietalbone. A cephalhematoma is a subperiosteal hemor-rhage occurring as a result of vessel rupture at birth. Itis generally not apparent at birth but is noted in thefirst day or two of life. It should be distinguished froma caput succedaneum. It is most commonly seen overthe parietal bones and more commonly over the rightparietal than the left. A caput succedaneum andcephalhematoma may occur concurrently.

Figure 4.8. Large left parietal cephalhematoma. Note

that the cephalhematoma is limited by suture linesbecause it is a subperiosteal hemorrhage. Cephal-hematoma occurs more commonly after prolongedprimigravida labor or forceps delivery especially in postterm infants where suture fusion makes the skull hardand unyielding.

Figure 4.9. Bilateral cephalhematomas in this infantdemonstrate very clearly the limitation of the cephal-hematoma by suture lines. Complications of cephal-hematomas include anemia, jaundice, infection, andunderlying skull fracture. In general, cephalhematomasresolve spontaneously over a period of weeks tomonths.

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Figure 4.10. Cephalhematoma with linear fracture of the skull.This occurs in 4 to 5% of infants with a cephalhematoma.

Figure 4.11. Calcification in a cephalhematoma giving thelesion an “egg shell” feel occurs as a result of deposition of calcium in the organizing blood. Periosteal new bone formsaround the perimeter of the cephalhematoma and this rimof calcification may be noted for several months.

Figure 4.12. Infected cephalhematoma inan infant with Escherichia coli sepsis.Osteomyelitis of the underlying parietalbone was present.

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Figure 4.13. The same infant asin Figure 4.12 with pus beingdrained from the infectedcephalhematoma (pyocephal-hematoma). The pus grew pureE. coli in culture. In generalit is recommended that cephal-hematomas not be aspirated, buton rare occasions, if the infantdevelops sepsis due to anyorganism, infection may occurin the cephalhematoma requir-ing drainage.

Figure 4.14. Radiograph of the skull of the sameinfant showing osteomyelitis of the parietal bone.

Figure 4.15. Massive scalp hemorrhage of thenewborn (subgaleal hemorrhage). When thereis rupture of the capillaries in the subaponeu-rotic area, there may be massive scalp hemor-rhage with spread over the entire scalp andmassive blood loss. Disseminated intravascularcoagulopathy may ensue rapidly. This condi-tion may occur as a result of a precipitousdelivery or poor application of a vacuumextractor. Note the massive soft tissue swelling.

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Figure 4.16. Frontal view of thesame infant who had a massive scalphemorrhage with a skull fracture.

Note the ecchymoses of the uppereyelids and marked swelling fromthe bridge of the nose extendingover the scalp. This is characteristicof a subgaleal hemorrhage as theaponeurosis of Galen attaches at theupper eyelids and has no attach-ment to the scalp until the nape of the neck and the sternocleidomas-toid muscle on the sides, hence themassive hemorrhage in the sub-galeal area of the head.

Figure 4.17. A massive scalp hemorrhage

following vacuum extractor delivery. Notethe limitation of bleeding at the nape of theneck and the sternocleidomastoid muscle.The bleeding into the subaponeurotic spacemay result in massive blood loss leading tohypovolemia and shock.

Figure 4.18. Hematoma of the rightcheek and mouth which occurred during adifficult spontaneous delivery.

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Figure 4.19. Trauma from a forceps delivery. Forcepsmarks on the cheek are fairly common. Rarely, a tran-sient facial palsy may be associated with this type of trauma and occasionally the forceps may actually trau-matize the skin, leading to ulceration.

Figure 4.20. Forceps mark following delivery.The pressure of the forceps blade may result indamage to the underlying tissue and, as in thisinfant, subcutaneous fat necrosis may occur.

Figure 4.21. Fetus born in acaul with a nuchal cord. Cordaround the neck once is presentin about 20% of deliveries and,in about 2% of deliveries, thereis a cord around the neck twice.This common finding generallydoes not cause problems unlessthe cord constricts the necktightly. (Klima, T.)

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Figure 4.22. Facial suffusion due to a nuchalcord.

Figure 4.23. Cord around the neck interfering with cir-culation. Note the petechiae of the face and head and thesubconjunctival hemorrhages. Petechiae are also seen ininfants in whom there is abnormal delay following deliv-ery of the head and neck before the trunk and shouldersare delivered. In subconjunctival hemorrhage a linear orlunar hemorrhage is often seen to the side of the iris.Petechiae and subconjunctival hemorrhages do not havethe same ominous significance as those on the trunk orlimbs and usually fade in the first few days of life.

Figure 4.24. Marked suffusion and bruising of theface as a result of a face presentation. This could beconsidered the equivalent of a caput with the face asthe presenting part. There is deep blue discolorationand swelling of the face and there may be consider-able disfiguration of the face which is of short dura-tion.

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Figure 4.25. Face presentation in a prematureinfant of 34 weeks gestation. Note the very markedecchymotic appearance of the face.

Figure 4.26. Face/brow presentation. Note themarked edema and ecchymoses over the faceand brow, particularly the left eye.

Figure 4.27. A brow presentationwith hyperextension of the head.This is the classic “militaristic atti-tude” with head back and chin out.Opisthotonos is excluded by lackof arching of the back. As this isthe baby’s “position-of-comfort” inutero, during the first few days post-natally the infant will be unhappy if an attempt is made to flex the head.After several days the infant willadopt a normal posture.

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Figure 4.28. In this infant who was a breech

presentation note the edema, bruising, andecchymosis. In breech presentations the perineum,buttocks, and thighs may be severely bruised.

Figure 4.29. This infant is another example of abreech presentation. Note the extended legs andthe equivalent of a caput over the right buttock,

which was the presenting part.

Figure 4.30. Bruising of male genitalia due to abreech presentation. Notethe marked swelling of thescrotum and penis. In rarecases testicular trauma mayoccur.

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Figure 4.31. Breech presentation in a female infantwith marked bruising and swelling of the genitalia.

Note the swollen labia majora and bruised labia minora.

Figure 4.32. Petechiae following a breechdelivery are frequently seen in otherwise nor-mal infants. They are of no consequence andimprove spontaneously in a few days.

Figure 4.33. Typical position-of-comfort of an infant who was a frankbreech presentation. Note the mildgenu recurvatum. This infant kepther legs in extension with the kneesflexed for several days. “Position-of-comfort” deformations are commonin breech presentations and improvein a few days. These infants shouldall be checked for congenital disloca-tion of the hip.

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Figure 4.34. The characteristic moldingof the head of an infant in a breech pre-sentation. The frontal view shows theoccipitofrontal head elongation along witha prominent occipital shelf and the neckappears long.

Figure 4.35. Lateral view of the head of the same infantshows the flattening of the vertex and the prominent

occipital shelf. The plane of flattening is directedupward and forward from the occipital protuberance,which is quite prominent. The characteristic headresults from prolonged pressure of the flexed headagainst the fundus of the uterus in breech presentation.

Figure 4.36. “Hangingneck” contusion in a difficultdelivery of a breech pre-sentation. The infant wasextremely depressed andrequired ventilatory support.

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Figure 4.37. Double footlingbreech presentation. The right legpresented through the cervical osfor 6 hours prior to delivery. Notethe marked ecchymoses and edema.This resolved spontaneously butthe infant developed hyperbiliru-binemia.

Figure 4.38. This mother had a history of adifficult previous abortion. With this preg-nancy she had a spontaneous rupture of the

uterus. On laparotomy a large perforationwas noted in the uterus through which theinfant’s lower extremities presented. Notethe compression edema and ecchymoses of the left leg and the gangrene of the rightfoot.

Figure 4.39. A close-up view of thegangrene of the right foot in the sameinfant.

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Figure 4.40. Subcapsular hematoma of the liver isgenerally a pathologic finding. It occurs most com-monly with breech delivery especially in prematureinfants. Occasionally, if bleeding persists, the capsuleof the liver ruptures and there is massive hemorrhageinto the peritoneal cavity. This usually occurs on thethird or fourth day of life. The same may occur in the

course of mismanaged artificial cardiopulmonary resus-citation. Rupture of the spleen is very rare and is morecommon with a transverse lie.

Figure 4.41. Transverse lie with a shoulderpresentation. Note the marked swelling andecchymoses of the right shoulder and upperextremity.

Figure 4.42. Compound presen-tation of the right arm, hand andvertex. Note the depression in theskull.

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Figure 4.43. The same infant with the arm and handplaced in its in utero position. Note the marked edemaof the right forearm and hand compared to that of theleft arm and hand. This occurred as the result of thecompound presentation.

Figure 4.44. In this infant the membranes ruptured 10days prior to delivery which was by cesarean birth forchorioamnionitis. There was difficulty in deliveringthe right arm which presented at the elbow. The handis normal; therefore this was not due to an amnioticband.

Figure 4.45. Intrauterine skull fracture (congenitalmolding of the skull) which occurs in cases of infantswith poor mineralization of the skull where themother has a prominent sacral promontory and has aprolonged labor or delivers precipitously. It resultsin a greenstick or depressed fracture of the skull.

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Figure 4.46. Radiograph of the skull of theinfant shown in Figure 4.45 showing adepressed fracture in a poorly mineralizedskull after a spontaneous vertex delivery. Inthese infants the fracture may improve spon-taneously but there is a question as towhether treatment should be passive or sur-gical lifting of the fracture is indicated.

Figure 4.47. Another example of an intrauterine skullfracture (also called congenital molding of the skull).

Figure 4.48. Radiograph of adepressed fracture of the skull follow-ing forceps trauma. A linear skull frac-ture may be seen underlying a cephal-hematoma or may occur from postna-tal trauma such as in an infant fallingfrom the bed to the floor.

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Figure 4.49. This infant had depression on both sidesof its skull from its intrauterine position. Note that thisis not traumatic and requires no treatment.

Figure 4.50. Cervical cord injury. This veryrare complication is invariably associated with

breech delivery. Note the crying infant lyingflat on the bed in the “pithed-frog” positionwith abdominal distention due both to lack of muscle tone and to an enlarged bladder.

Figure 4.51. Radiograph of the neckshowing the cervical cord and spinalinjury following breech delivery. Notethe fracture dislocation and separa-tion involving C5 and C6.

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Figure 4.52. Congenital torticollis is usuallynot apparent at birth but within the firstweek a swelling is noted over the sternocl ei-domastoid muscle (stenomastoid tumor).T

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