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Oligohydramnios Sequence Revisited in Relationship to Arthrogryposis, with Distinctive Skin Changes Judith G

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RESEARCH ARTICLE Oligohydramnios Sequence Revisited in Relationship to Arthrogryposis, With Distinctive Skin Changes Judith G. Hall* Departments of Medical Genetics, Pediatrics, University of British Columbia, BC Children’s Hospital Vancouver, British Columbia, Canada Manuscript Received: 3 September 2013; Manuscript Accepted: 1 July 2014 Thirty cases of arthrogryposis associated with longstanding oli- gohydramnios were identified among 2,500 cases of arthrogry- How to Cite this Article: posis (1.2%) and were reviewed for clinical features and natural Hall JG. 2014. Oligohydramnios sequence history.Nonehadrenal agenesis or renal disease. Twenty-twohad revisited in relationship to arthrogryposis, a history of known rupture of membranes. Only 50% had pul- with distinctive skin changes. monary hypoplasia at birth and only two died (7%). Sixty percent (18/30) seemed to have their multiple congenital contractures Am J Med Genet Part A 164A:2775–2792. (MCC) primarily on the basis of compression related to the longstanding oligohydramnios and responded well to physical therapy. On average they did not have intrauterine growth author over a 35-year period. These cases come from clinic visits restriction. “Potter” facies and remarkable skin changes were and consultations, as well as correspondence with physicians and present in all. An excess of males was observed in spite of the lack families. Thirty individuals with longstanding oligohydramnios of genitourinary anomalies. Ó 2014 Wiley Periodicals, Inc. (average five months) were identified. Of these individuals, 20/ 30 were personally examined in relation to their congenital con- Key words: arthrogryposis; multiple congenital contractures; tractures and other clinical signs. These 30 affected individuals are abnormal hair pattern; amniotic fluid leakage; attempted clearly a biased collection, not representative of the normal or termination of pregnancy; chorionic villus sampling (CVS) arthrogrypotic population; however, they are being reported in compression; deformation; dimple; excess skin; large ears; male order to raise awareness of their findings and present some inter- excess; multiple congenital anomalies; oligohydramnios; Potter esting questions that they raise. Family, pregnancy, and natural syndrome; pulmonary hypoplasia; redundant skin; renal agenesis; histories were also reviewed. The records and photographs of the 30 uterine anomaly; webbing individuals were examined, tabulated, and compared to the overall group of cases with arthrogryposis (see Table I). INTRODUCTION RESULTS WITH COMMENTARY Oligohydramnios (decreased amniotic fluid for gestational age) Demographics was identified in the early 1900s by Pilgrim when it was observed Both the time of year of birth and the parental age were not unusual that it lead to changes in the placental surface (placenta nodosum) compared to the other cases of arthrogryposis. Regarding the length [Blanc, 1961]. Renal agenesis was later recognized to be frequently of pregnancy: in one-third of these individuals, the births clustered associated with oligohydramnios and multiple congenital contrac- during the two weeks before and after term. The one third who were tures by Bates [1933], and further expanded upon by Potter [Potter, born before 36 weeks, were usually premature related to induction 1946a,b]. This paper is a review of 30 cases of arthrogryposis (multiple congenital contractures) all without renal agenesis, which experienced longstanding oligohydramnios. The purpose of this Conflict of interest: The author has no known conflicts of interest. paper is to examine the relationship of oligohydramnios to arthrog- Ã ryposis (multiple congenital contractures) and the secondary and/ Correspondence to: Judith G. Hall, OC, MD, FRSC, FCAHS, Department of Pediatrics, or tertiary effect on fetal skin. British Columbia’s Children’s Hospital, 4500 Oak Street, Room C234, Vancouver, BC, Canada, V6H 3N1. METHODS E-mail: [email protected] Article first published online in Wiley Online Library Clinical information on 2,500 individuals with arthrogryposis (wileyonlinelibrary.com): 26 August 2014 (multiple congenital contractures) have been collected by the DOI 10.1002/ajmg.a.36731 Ó 2014 Wiley Periodicals, Inc. 2775 2776 TABLE I. 30 Cases of Arthrogryposis With Oligohydramnios Weeks of Placenta Pregnancy; Weeks of and Oligohydra- Intervention or Complications Gestation Size at Umbilical Parental Striking Limb Pulmonary Prematurely No. mnios Oligo noted of Pregnancy at Birth Delivery Birth Cord Gender Age M/F Skin Position Mentation at Birth Related II˚ Other Organs Final Dx 1 ROM 8 weeks On seizure 32 Breech; 1.4 kg NR F 20/28 Deep Extended Bright Retinopathy of - ATP and medication; C-section dimples arms prematurity chronic ROM and oligo legs; Fx at birth; hyperext- ended 2 ATP 8 weeks ATP; UTI Rx; 31 Breech; 2.4 kg NR M 16/17 Posterior Flattened Hyperactive; Pulmonary Pneumothorax; Hypospadia; ATP with possible twin C-section hair with distal normal hypoplasia required cryptorchidism; chronic tongue of limbs; hyperalime- diathesis and oligo hair on mild ntation hypoplasia of forehead; flexion abdominal edema; muscle loose skin 3 ROM 9 weeks ARTs (Clomid); 28 Vertex; 2 lb 1 oz Long cord M 32/30 Edema; Generalized Hyperactive Croaky voice; - abndominal wall ROM; chronic 9 weeks C-section deep flexion; hypoplastic muscle; oligo bleed; US dimples; dislocated limbs bilateral fluid around loose hip inguinal mouth skin hernias 4 ATP 11 weeks ATP; maternal 31 Breech; 1.6 kg NR F 31/30 Deep Generalized Intellectual Respiratory - Dilated ATP with herpes C-section dimples; flexion delay, CP distress ventricle; chronic extra skin; at birth pulmonary oligo webs stenosis; trismus 5 Connective Early Back pain; 40 NR 3.3 kg NR M 21/- Loose extra Generlized Normal NR - GI reflux; short Connective tissue; leakage skin; loose flexion palpebral tissue, ROM throughout joints fissure; ROM; arachnodactyly; with cryptorchidism chronic oligo 6 Early CVS 12 weeks Oligo; post 35 Breech; 1.7 kg Amnion F 37/- Cutis Generalized Apparently Mild - Umbilical hernia; Chronic oligo leakage; CVS; IUGR C-section nodosum; mamorata; flexion; normal pulmonary cord wrapping secondary ROM long cord dimples spatuated hypoplasia; on leg to CVS around hands thin neck; diaphragm circum- vallate placenta 7 ATP 12 weeks ATP; GDM 36 Vertex; 2.2 kg 3 vessel F 40/41 Dimples; Generalized Slow Early Periventricular Required G-tube; ATP with fibroid C-section cord; flattened flexion at respiratory echogenic trismus chronic normal face birth; problem oligo placenta flattened AMERICAN JOURNAL OF MEDICAL GENETICS hands; Fx x2 at birth 8 ROM 12 weeks Bleeding 1st 30 Breech; 1.1 kg NR M 22/41 Redundant Extended Alert Pulmonary Retinopathy of Abdominal ROM; chronic and 2nd; vaginal skin arms; hypoplasia prematurity muscle; oligo ROM flexed pachygyria; hip and mild knees; ventricular Fx leg enlargement at birth 9 ROM 13 weeks ROM 32 C-section; 1.9 kg NR M 32/23 Redundant Generalized Mild develop- Pulmonary Chronic Needed GJ tube Prematurity; fetal skin; flexion; Fx mental hypoplasia respiratory for feeding; chronic distress droopy related to delay insufficiency decreased lung; cheeks; osteopor- facial ROM, deep osis at movement chronic dimples birth oligo 10 ROM 13 weeks " MSAFP; 26 Breech; 900 g Subchorionic M 24/- Scar on Generalized Normal Pulmonary Retinopathy of Shawl scrotum; ROM; chronic ROM C-section separation leg; loose flexion; hypoplasia; prematurity heart murmur; oligo 13 weeks skin; dislocated respiratory sacral dimple; hyperexte- hip distress bifid uvula; ndable; inguinal hernia webs 11 ROM 13 weeks ARTs triplet; 38.5 C-section MCDA; 2 F - Redundant Generalized Developmental Chronic - Moebius; ROM; chronic IUGR; GDM vessel skin; deep flexion delay lung; " AP myopathy; oligo cord dimples G tube; PART A bicuspid aorta HALL TABLE I. (Continued) Weeks of Placenta Pregnancy; Weeks of and Oligohydra- Intervention or Complications Gestation Size at Umbilical Parental Striking Limb Pulmonary Prematurely No. mnios Oligo noted of Pregnancy at Birth Delivery Birth Cord Gender Age M/F Skin Position Mentation at Birth Related II˚ Other Organs Final Dx 12 ROM 16 weeks Fibroid; 40 Vaginal; 7 lb 12 oz Placenta F 32/38 Patch of Generalized Bright " AP; short - Nasal voice; ROM; chronic trauma vertex previa unusual flexion; of breath ptosis; oligo to abdomen; hair; vertical neck pain; 16 weeks; webbing; talus decreased ARTs; deep facial leakage dimples; movement keloids 13 ROM 16 weeks movement; 36 Breech, 4 lb 9 oz - M 29/32 Dramatic Generalized Bright - - Hemangioma; ROM, chronic oligo C-section dimples; flexion upper, oligo 2 hair sacral whorls dimple with hair; tethered cord 14 ROM 18 weeks Bedrest; ROM 34 C-section 4 lb Long cord F 29/31 Extra skin R side Okay Wide spaced - facial ROM; chronic flexion; nipples; movement oligo flattened early early hands pulmonary and feet distress; " AP 15 ROM 18 weeks UTI; ROM 34 Vaginal; 3 lb 8 oz NR F 27/29 Hirsuit; thin Flexed legs; Bright - Hemangioma ROM; chronic breech skin; dislocated chest oligo extra skin; hip; short webbing toes 16 ROM 20 chorionic Twin died at 27 Vertex; 1.0 kg Abruptio F 29/- Many Generalized Bright Severe Retinopathy of Femoral and ROM, chronic birth C-section placenta; dimples; flexion pulmonary; prematurity inguinal oligo; DZ cord webbing; hypoplastic hernias; twin normal depigmented nipples myopia areas of neonatal? skin Seizures 17 ROM 20 chorionic; IUGR in 3rd 37 Vaginal 4 lb 2 oz NR M 28/30 Two hair Generalized Normal Hoarse voice;
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